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Pheochromocytoma in an 8‐year‐old patient with multiple endocrine neoplasia type 2A: Implications for screening
Author(s) -
Rowland Kathryn J.,
Chernock Rebecca D.,
Moley Jeffrey F.
Publication year - 2013
Publication title -
journal of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.201
H-Index - 111
eISSN - 1096-9098
pISSN - 0022-4790
DOI - 10.1002/jso.23378
Subject(s) - medicine , pheochromocytoma , multiple endocrine neoplasia , multiple endocrine neoplasia type 2 , endocrine system , general surgery , oncology , hormone , mutation , genetics , germline mutation , gene , biology
Childhood pheochromocytoma in the setting of multiple endocrine neoplasia type 2 (MEN2) remains rare and has not been reported under the age of 12. We present an 8‐year‐old female with known MEN 2A, C634Y RET mutation, diagnosed with a 6 cm pheochromocytoma requiring laparoscopic adrenalectomy. Given this patient's age at diagnosis, screening guidelines should recommend annual screening beginning at age 8 for patients with MEN 2B or MEN 2A codons 630 or 634 RET mutations. J. Surg. Oncol. 2013 108:203–206 . © 2013 Wiley Periodicals, Inc.